title: [A case of neuro-neutrophilic disease presenting with 5 months' with cognitive decline, meningoencephalitis, and marked systemic inflammatory findings, and diagnosed with brain biopsy].
abstract: A 63-year-old man was admitted to our hospital with cognitive decline. On admission, he had a fever and mild cognitive dysfunction, suggesting chronic meningoencephalitis. Apart from a mild increase in serum C-reactive protein level and marked neutrophilia, laboratory findings were unremarkable. Brain magnetic resonance (MR) imaging showed multiple small T2-hyperintense lesions in the white matter. Systemic evaluations for infectious organisms, autoantibodies, and malignancy were all negative. For 5 months we conducted therapeutic trials of various antibacterial, antifungal, and antituberculous drugs, but these were completely ineffective, and both meningoencephalitis and inflammatory signs persisted. Repeated brain MRI during the clinical course showed growth of the white matter lesions and progressive cerebral atrophy. C11-methionine positron emission tomography demonstrated a bright focus in the right frontal lobe, and this was biopsied. Key neuropathological findings were neutrophilic infiltration in the subarachnoid space and the frontal lobe without necrotic angiitis. These findings confirmed the diagnosis of neuro-neutrophilic disease, although skin tissue findings characteristic of Sweet disease and a B51, B54, or Cw1 HLA-profile were absent. After intravenous bolus administration of steroid and prolonged oral steroid therapy, fever and inflammatory signs diminished and cognitive symptoms improved.
authors.affiliation: Department of Neurology, Faculty of Medicine, Saitama Medical University.
authors.forename: Yasuko
authors.initials: Y
authors.lastname: Ohe
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Nakazato
authors.forename: Keisuke
authors.initials: K
authors.lastname: Ishizawa
authors.forename: Ichiro
authors.initials: I
authors.lastname: Deguchi
authors.forename: Naotoshi
authors.initials: N
authors.lastname: Tamura
authors.forename: Nobuo
authors.initials: N
authors.lastname: Araki
mesh_terms: D000368:Aged; D001528:Behcet Syndrome / Q000175:diagnosis; D001706:Biopsy; D001921:Brain / Q000473:pathology*; D003072:Cognition Disorders / Q000175:diagnosis*; D003937:Diagnosis, Differential; D006801:Humans; D007249:Inflammation / Q000175:diagnosis; D008297:Male; D008590:Meningoencephalitis / Q000175:diagnosis*; D016463:Sweet Syndrome / Q000175:diagnosis* / Q000473:pathology